Idiopathic Pleuroparenchymal Fibroelastosis as a Rare Entity within Idiopathic Interstitial Pneumonias

Pleuroparenchymal fibroelastosis (PPFE) is an upper lobe predominant progressive fibrosis with specific pathologic findings including visceral pleural thickening, subpleural elastosis, and intraalveolar collagenous fibrosis. 

PPFE is now recognized by the American Thoracic Society (ATS)/European Respiratory Society (ERS) in their most recent statement on idiopathic interstitial pneumonia in the category of rare idiopathic interstitial pneumonias. Many of the PPFE cases are associated with underlying conditions but the majority is idiopathic. We aimed to present the clinical, radiological and histopathological features of PPFE in a case diagnosed as IPPFE. Read more>>>>>>>>>>>>

Controversial Role of Iron in Colorectal Carcinogenesis

Colorectal Cancer (CRC) is the third most common cancer in males and the second in females worldwide with over 1.2 million new cases and over 600,000 estimated deaths in 2008. In the United States, it is estimated that 142,820 new cases of CRC withover 50,000 deaths occur annually. The development of CRC has been shown to be related to individual’s genetics and environmental factors. 

The genetic effect on the development of sporadic CRC is reflected by different incidences occurring based on age, sex, and ethnicity. About 10-15 % of CRC are solely attributable to genetic abnormalities. Hereditary CRC syndromes such as familial adenomatous polyposis (FAP) and hereditary non polyposis colorectal cancer (HNPCC) are two of these examples. The development of sporadic CRCs, comprising the majority of cases, has been linked to many environmental factors. Read more>>>>>>>>>>>>>>

Secretory Carcinoma of the Breast in an Elderly Woman

Secretory carcinoma is a rare low-grade breast carcinoma, characterized by the presence of large amounts of intracellular and extracellular secretory material and composed of cells with granular eosinophilic or amphophilic cytoplasm. This tumor frequently occurs in either children or adolescents. 

Though it is considered rare in adults, it is potentially more aggressive than in children. Originally this tumor was described in children, but it is now known to occur in adults of both sexes including a substantial number of post-menopausal women. We present the case of a 66-years-old woman with juvenile secretory carcinoma of the breast treated by mastectomy and axillary node dissection.

Case Report: A 66-year-old female patient presented with a subareolar mass of the right breast since 5 years. The mass suddenly increased in size with no evidence of palpable axillary lymph nodes. Serum tumor markers and other routine blood test were normal. The liver ultrasonography and chest X-ray were negative for metastases. A radical mastectomy with axillary node dissection was decided and performed. Grossly, the mass was circumscribed, measured 50 × 35 mm and had white glistening cut section. Microscopic examination revealed the classical features of secretory carcinoma with microcystic, papillary and solid patterns with abundant intra and extracellular PAS-positive secretory material. Read more>>>>>>>>>>>>>>>

A Granulomatous Hypophysitis "Consequent" to a Silent ACTH Cell Adenoma

Pituitary inflammatory lesions are uncommon: a granulomatous inflammatory reaction can be caused by systemic tuberculosis, syphilis or fungal infections, being usually an incidental autopsy finding. They come to the pathologist more frequently when they are at an advanced stage, producing a mass effect and/or hypothalamic. However, the most enigmatic form of granulomatous hypophysitis is idiopathic. Exceptional is the association of this inflammatory lesion with adenomatous neoplastic one. The aim of our report was to make assumptions on the pathogenesis of this unlikely association.

Case Report: We report the case of a 50-year-old male with a history of frontal headache lasted for years. In the last three years he had noticed an unexplained weight gain, decreased libido and sporadic visual defects. For this reason, he went to an eye doctor who suggested to make a magnetic resonance (MRI) of the brain which revealed an intrasellar tumor with slight suprasellarextension, displaying areas of hemorrhage and necrosis, most likely a pituitary macroadenoma. A computerized examination of the visual field highlighted a temporal hemianopsia of the left eye.   Read more>>>>>>>>>>>>>

Two Different Aspects of Pleuroparenchymal Fibroelastosis: A Disease of Pulmonary Fibrosis, and of the Chest Wall

In 2004, Frankel et al. reported five cases with upper-lobe-dominant pulmonary fibrosis that progressed gradually to respiratory insufficienc. As the histology comprised subpleural elastosis, intraalveolar collagenous fibrosis with septal elastosis and collagenous thickening of the visceral pleura, they named this unique disorder pleuroparenchymal fibroelastosis (PPFE). In 2013, PPFE of unknown etiology (idiopathic PPFE, IPPFE) appeared as one of the forms of idiopathic interstitial pneumonias (IIPs) in the international classification of these diseases. 

Since then, there have been increasing numbers of papers on PPFE. Although IPPFE is categorized as a rare form of IIP, it might not be as rare as reported previously. IPPFE, as a form of chronic fibrosing interstitial pneumonia, has a similar clinical course to that of idiopathic pulmonary fibrosis (IPF), and could be confused with IPF in clinical practice. Read more>>>>>>>>>>>>>>>